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In this case report, we highlight a rare case of palindromic rheumatism (PR) presenting as polymyalgia rheumatica (PMR). Many challenges and complexities are associated with diagnosing and treating PR. Literature reviews showed only a few case reports of this unique presentation. PR has a distinct presentation that often goes unnoticed and is misinterpreted by medical professionals. A more thorough clinical approach is required to identify and treat this condition. We hope sharing such uncommon cases will help the medical community better understand PR and develop improved diagnostic and therapeutic options. This case also demonstrates the need for further research to better understand the pathogenesis of this uncommon condition.

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Palindromic rheumatism (PR) is a type of cryptogenic paroxysmal arthritis. Several genes may be involved in PR pathogenesis; however, conducting comprehensive case-control genetic studies for PR poses challenges owing to its rarity as a disease. Moreover, case-control studies may overlook rare variants that occur infrequently but play a significant role in pathogenesis. This study aimed to identify disease-related genes in Japanese patients with PR using whole-genome sequencing (WGS) and rare-variant analysis. Genomic DNA was obtained from two familial cases and one sporadic case, and it was subjected to WGS. WGS data of 104 healthy individuals obtained from a public database were used as controls. We performed data analysis for rare variants on detected variants using SKAT-O, KBAC, and SKAT, and subsequently defined significant genes. Significant genes combined with variants shared between the cases were defined as disease-related genes. We also performed pathway analysis for disease-related genes using Reactome. We identified 2,695,244 variants shared between cases; after excluding polymorphisms and noise, 74,640 variants were detected. We identified 540 disease-related genes, including 1,893 variants. Furthermore, we identified 32 significant pathways. Our results indicate that the detected genes and pathways in this study may be involved in PR pathogenesis.

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BACKGROUND: Four criteria have been proposed for the diagnosis of palindromic rheumatism (PR), including those of Hannonen et al., Passero and Barbieri, Guerne and Weisman, and Gonzalez-López. But none of these criteria has been validated. In this research, we investigated the performance of these diagnostic criteria for diagnosing PR. METHODS: In this study, PR and control groups were consecutively recruited from a prospective cohort of intermittent arthritis. Inclusion criteria for PR group were diagnosing PR by an expert rheumatologist, age ≥ 18, having at least 6 months follow-up, and ruling out of other causes of intermittent arthritis. These criteria were applied to both groups. Sensitivity, specificity, positive predictive value, negative predictive value, diagnostic odds ratio (DOR), and Youden's index were calculated for each criteria. RESULTS: This study included 197 consecutive subjects diagnosed with PR and 208 subjects with a diagnosis other than PR. The sensitivity of Hannonen et al. criteria was higher than the Gonzalez-Lopez, Guerne and Weisman, and Pasero and Barbieri criteria (96.4% versus 95.4%, 79.2%, and 35.5%, respectively). The specificity of the Pasero and Barbieri criteria was higher than the other criteria. Hannonen al. criteria with a DOR of 325.7, had the highest DOR. In descending order, the best accuracy belonged to Hannonen et al., Gonzalez-Lopez, Guerne and Weisman, and Pasero and Barbieri criteria (94.3%, 94.1%, 86.4%, and 66.9% respectively). CONCLUSION: This study showed that the Hannonen et al. and Gonzalez-Lopez criteria have a better performance in diagnosing PR. Key Points • The sensitivity of Hannonen et al. criteria and the specifity of Passero and Barbieri criteria are higher than other proposed criteria for diagnosis of palindromic rheumatism. • Hannonen et al. criteria with a sensitivity of 96.4%, specifity of 92.3% and accuracy of 94.3% has the best performance in diagnosis of palindromic rheumatism between existing diagnostic criteria for palindromic rheumatism.

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OBJECTIVE: To determine whether there is a correlation between vitamin D levels and palindromic rheumatism (PR) as an at-risk phenotype of rheumatoid arthritis (RA). METHODS: A total of 308 participants were enrolled in this cross-sectional study. We recorded their clinical characteristics and performed propensity-score matching (PSM). Serum 25(OH)D3 levels were determined via enzyme-linked immunosorbent assay. RESULTS: Our PSM resulted in 48 patients with PR and 96 matched control individuals. The multivariate regression analysis we performed after the PSM did not show a significant increase in PR risk in patients with vitamin D deficiency/insufficiency. There was no significant correlation between levels of 25(OH)D3 and frequency/duration of attacks, number of joints affected, and duration of symptoms before diagnosis (P ≥ .05). Mean (SD) serum levels of 25(OH)D3 in patients with and without progression to RA were 28.7 (15.9) ng/mL and 25.1 (11.4) ng/mL, respectively. CONCLUSION: Based on the results, we found no clear association between vitamin D serum levels and the risk, severity, and rate of PR progressing into RA.

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Palindromic rheumatism (PR) is a unique syndrome considered a prelude to rheumatoid arthritis (RA). It is characterized by recurrent, unpredictable episodes of joint inflammation and distinct clinical features. Unlike RA, PR episodes are brief and reversible, involving sudden-onset joint pain, swelling, and erythema. The exact etiology and diagnostic criteria of PR remain elusive, but it often shares autoantibodies with RA, leading patients to transition from PR to RA. The management of PR is multifaceted and empirical, involving various treatment modalities such as non-steroidal anti-inflammatory drugs (NSAIDs), colchicine, and disease-modifying anti-rheumatic drugs (DMARDs). However, the relationship between obesity and PR remains underexplored. This case presents a 52-year-old woman, who presented to our hospital with recurrent and debilitating arthritis episodes since 2016. Initially affecting her wrists and later extending to her knees, shoulders, and back, these episodes severely impaired her daily activities. Despite a diagnosis of RA in 2019, supported by a positive rheumatoid factor (RF) test, and subsequent DMARD treatment, her symptoms persisted. In 2022, during her examinations at our hospital, the distinctive pattern of intermittent symptoms accompanied by damage-free joints was unveiled, suggesting a potential diagnosis of palindromic rheumatism. Notably, this case highlights the potential association between obesity and PR, as the patient's decision to undergo bariatric surgery in 2022 led to substantial weight loss of over 36 kg. This weight reduction yielded remarkable improvements in her condition, resulting in reduced frequency and severity of PR attacks. As a consequence, her medication regimen was simplified, emphasizing the therapeutic role of weight management in PR. This case paves the way for further research into the relationship between obesity, PR, and non-pharmacological interventions in PR management.

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BACKGROUND: As a new immunomodulator for rheumatoid arthritis, iguratimod (IGU) also has therapeutic potential in other immune diseases. In this study, we determined the effects of IGU on disease control in patients with palindromic rheumatism (PR). METHODS: Patients with PR were divided into Control group (Ctrl group) and an IGU treatment (IGU group) groups. Drug efficacy was evaluated according to the frequency of PR attacks (monthly), the visual analog scale (VAS) score of patient pain, and clinical symptoms. RESULTS: The drug positivity and disease control rates of the IGU group (100.00% and 90.91%, respectively) were significantly higher than those of the Ctrl group (61.11% and 5.56%; p = .002 and p < .001, respectively). The median number of PR flares and the VAS score of patients in the Ctrl group decreased from 3.00 (1.00-15.00) to 0.83 (0.00-12.00) and from 5 (4-6) to 4 (1-6), respectively. In the IGU group, the median number of PR attacks decreased from 4.50 (2.00-15.00) to 0.00 (0.00-0.33), and the VAS score decreased from 5 (4-6) to 0 (0-2). The IGU group exhibited a significant reduction in PR flare frequency and improvement in the VAS value (p < .001 and p < .001, respectively). CONCLUSION: Our study is the first to describe the efficacy of IGU in PR treatment. IGU can significantly reduce the number of PR flares and improve the clinical symptoms of patients with PR.

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In recent years rheumatologists have begun to shift focus from early rheumatoid arthritis (RA) to studying individuals at risk of developing the disease. It is now possible to use blood, clinical and imaging biomarkers to identify those at risk of progression before the onset of clinical synovitis. The use of imaging, in particular ultrasound (US) and magnetic resonance imaging (MRI), has become much more widespread in individuals at-risk of RA. Numerous studies have demonstrated that imaging can help us understand RA pathogenesis as well as identifying individuals at high risk of progression. In addition, imaging techniques are becoming more sophisticated with newer imaging modalities such as high-resolution peripheral quantitative computed tomography (HR-pQRCT), nuclear imaging and whole body-MRI (WB-MRI) starting to emerge. Imaging studies in at risk individuals are heterogeneous in nature due to the different at-risk populations, imaging modalities and protocols used. This review will explore the available imaging modalities and the rationale for their use in the main populations at risk of RA.

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OBJECTIVE: The goal of treatment in palindromic rheumatism (PR) is to control the attacks and prevent disease evolution to chronic arthritis. Conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) including antimalarial and methotrexate cannot control attacks in all patients. METHODS: In this retrospective study, we assessed the efficacy of leflunomide in patients with PR who had an inadequate response to DMARDs. In this study, patients who had a diagnosis of PR and were treated with leflunomide because of active disease despite treatment with csDMARDs for at least 6 months were included. Remission was defined as no attacks for 3 months and prednisolone dose ≤5 mg/d. Leflunomide treatment failure was defined as failure to achieve remission, the need to add other DMARDs for controlling attacks and disease progression to chronic arthritis during treatment with leflunomide. RESULTS: Ten cases with active disease despite treatment with hydroxychloroquine and methotrexate and low-dose prednisolone treated with leflunomide were included in the study. During the 12.6 ± 7.5 months of treatment with leflunomide, the frequency of attacks significantly decreased. Complete and partial remission were achieved in 90% of patients. CONCLUSION: Our results indicate that leflunomide controls PR attacks and it might be a new option for patients with PR.

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OBJECTIVE: Palindromic rheumatism (PR) is characterized by self-resolving and short duration attacks of arthritis/periarthritis. The present study was performed to report the results of PR treatment with methotrexate (MTX). METHODS: We reviewed the charts of 152 patients with diagnosis of PR. Inclusion criteria were diagnosis of PR according to the criteria of Weismann, age ≥16, active disease and treatment with MTX for at least 6 months. Disease outcome was assessed by reaching remission and prevention of disease evolution to chronic arthritis. Remission was defined as stopping the attacks for 12 weeks and prednisolone dose ≤5 mg/d. MTX treatment failure was defined as failure to achieve remission, the need to add other disease-modifying antirheumatic drugs and disease progression to chronic arthritis. RESULTS: Fifty-nine patients were included in the study. Median duration of follow-up was 43 months. Attacks were controlled in 89.8% of patients. In 80% of the patients remission occurred during 12 months after starting treatment with MTX. Treatment failed in 20.3% of patients. Wrist joint involvement and positive rheumatoid factor (RF) were significantly more common in the MTX treatment-failed group. In RF positive patients evolution to rheumatoid arthritis was more common than in RF negative patients. No significant differences were observed in remission rate and evolution to rheumatoid arthritis in anticitrullinated C peptide positive and negative patients. CONCLUSIONS: The present study, demonstrated the efficacy of MTX in controlling PR in seropositive and seronegative patients over a median of 43 months of treatment.

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Present study was conducted to investigate smoking status in palindromic rheumatism (PR) patients compared to healthy individuals as well as to assess the effect of smoking on clinical features and outcomes of PR. One hundred and forty-six patients with diagnosis of PR and 346 healthy controls were included in this study. Demographic, clinical, and laboratory characteristics and the smoking history of PR patients at the cohort entry were obtained from patients' records. Demographic and smoking history of the control group were obtained by direct interview. In order to reduce heterogeneity between the studied groups, propensity score matching (PSM) analyses was performed. Matching was achieved by considering age, gender, educational status, and marital status. After PSM, we carried out a multivariate analysis with PR as the main outcome variable, ever smoking as the main predictor variable and age, gender, educational status, and marital status as covariates. PSM resulted in 123 PR patients and 246 matched controls. Multivariate analysis did not show a significant increase in the risk of PR in ever smokers. Seventy-six patients were anti-citrullinated protein/peptide antibody positive (ACPA-positive). Multivariate logistic regression showed a significant increase in the risk of PR in ACPA-positive ever smokers. Except lower sustained remission rate in ever smokers, no significant differences were observed in clinical manifestations and outcomes of PR between ever and never smokers. In conclusion, smoking is a risk factor for ACPA-positive PR.

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INTRODUCTION: MEFV mutations have been documented in patients with palindromic rheumatism (PR) who do not meet FMF criteria, and RF and ACPA positive RA may start with PR. OBJECTIVE: To analyze the clinical phenotype and disease evolution of patients with intermittent, palindromic-like (PL) arthritis seen in our Arthritis Unit according to the RF, ACPA and MEFV mutation status. METHODS: MEFV genotyping was done in 76 patients with PL arthritis as defined by predominantly short attacks (≤7days) and a relapsing course. Characteristics of arthritic episodes, RF and ACPA positivity, and the colchicine response were retrospectively collected. Patients were stratified and evaluated according to MEFV mutations and/or positive autoantibodies (ACPA and/or RF). RESULTS: Among the patients, 26.3% (20/76) had a MEFV mutation and 23 (30%) were ACPA and/or RF positive. MEFV mutations and/or autoantibody status allowed four PL arthritis patients to be distinguished: group I (MEFV+), with younger age of onset, short duration attacks (<3days), mainly located in the knee, more frequent non-articular manifestations (fever, pericarditis or abdominal pain) and good response to colchicine; group II (autoantibody+) is older than group I, with the same frequency of short attacks, but the most affected joints were the wrists and small joints of hands: 48% met RA classification criteria during follow-up and were taking DMARDs; group III (MEFV- and autoantibody-) was the most frequent (48%) and clinically heterogeneous group; 51% had attacks lasting>3days, and 15 patients developed criteria of immune-mediated inflammatory, autoinflammatory or infectious diseases. Group IV (MEFV+ associated with preexisting immune-inflammatory disease), was associated with very short attacks, like groups I and II, superimposed or coincident with definite immune-inflammatory disease, including seropositive RA, with good response to colchicine. CONCLUSIONS: Patients with PL arthritis can be classified in four groups according to the presence or not of MEFV mutations and ACPA/RF antibodies with a different clinical evolution and therapeutic response.

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BACKGROUND: Rosai-Dorfman-Destombes (RDD) disease, is a rare proliferative and inflammatory disorder of non-Langerhans cell histiocytes. CASE PRESENTATION: We report a 35-year-old woman, who originally presented with recurrent episodes of lower extremity joint/bone pain and chronic nasal stuffiness and congestion. Her worsening nasal congestion was due to an obstructing nasal cavity lesion which was subsequently biopsied. Pathology was consistent with RDD. 18F-FDG PET images demonstrated intense uptake in the paranasal sinuses and a large pelvic lymph node. Focal osseous lesions with intense 18F-FDG uptake were also observed in the lower extremity, corresponding to areas of peri-articular pain. Rheumatologic work-up was consistent with palindromic rheumatism. She was diagnosed with immune-related disseminated RDD, presenting as palindromic rheumatism. CONCLUSIONS: This is the first case of RDD presenting as palindromic rheumatism. RDD should be considered as a possible but rare diagnosis in young patients with sinus-related symptoms and lymphadenopathy. The disease can on rare occasions be disseminated and can also present as immune-related RDD, such as in this patient.

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Palindromic rheumatism (PR), a unique clinical entity, has a characteristic clinical presentation with a relapsing/remitting course. It is established that most patients with PR evolve to chronic disease, of which rheumatoid arthritis (RA) is by far the most common. The relationship between PR and RA is unclear, with similarities and differences between the two, and not all patients evolve to RA in the long-term. Therefore, PR is clearly a pre-RA stage for most, but not all, patients. Autoimmunity plays a substantial role in PR, with the same characteristic autoantibody profile observed in RA, although with some differences in the immune response repertoire. Autoinflammation may also be relevant in some cases of PR. Prognostic factors for RA progression are identified but their exact predictive value is not clear. There are several unmet needs in PR, such as the diagnostic criteria and clinical case definition, the pathogenic mechanisms involved in the unusual clinical course, and the evolution to RA, and our understanding of the therapeutic strategy that could best avoid progression to persistent and potentially destructive arthritis.

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INTRODUCTION: Palindromic rheumatism (PR) is a form of relapsing/remitting arthritis that may evolve to chronic rheumatic disease, mainly rheumatoid arthritis (RA). The exact nature of PR is unclear, as it may be considered a disease in itself, an abortive form of RA or just a pre-RA stage. AREAS COVERED: The authors review the most relevant epidemiological and clinical aspects of PR, especially the pathogenetic role of autoimmunity in PR, with most patients having a characteristic autoantibody profile similar to that observed in RA. The role of autoinflammation is also discussed. A literature review on the rate of RA progression and its prognostic factors was analyzed. Data on the efficacy of drug therapies used to treat PR are presented. PubMed was searched using the terms 'palindromic rheumatism' OR 'palindromic arthritis'. EXPERT OPINION: PR is a disease entity with a close but unclear relationship with RA. In PR there is an unmet need, which is to clarify the clinical spectrum and elucidate the risk factors for evolution to RA. The role of autoimmunity and the autoinflammatory component should be investigated. Since most patients evolve to RA, PR may display a unique therapeutic opportunity to avoid this evolution.

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Objectives: Palindromic rheumatism (PR) is characterized by repetitive, afebrile episodes of acute arthritis and peri-arthritis. The aim of this study was considering the long-term outcomes of patients with PR who were treated with tight control strategy using Disease-modifying anti-rheumatic drugs (DMARDs). Methods: We reviewed the charts of 106 patients diagnosed with PR who were referred to the Connective Tissue Diseases Research Center (CTDRC). We recruited all the patients diagnosed with PR according to the criteria of Hannonen. They visited the CTDRC clinic regularly and were treated with hydroxychloroquine and low dose prednisolone because of active episodes of PR. In cases that the attacks did not come under control in 3–6 months, methotrexate was added or replaced and the dose was increased up to 25mg/week. In resistant cases, sulfasalazine was added, followed by the addition of leflunomide and then azathioprine. Disease outcome was evaluated by getting complete or partial remission and prevention of disease evolution to rheumatoid arthritis (RA) or other inflammatory connective tissue diseases. Results: This study included 92 patients with PR who were treated with DMARDs. Attacks were controlled completely or partially in 76 (82.6%) patients. Medications free remission was obtained in 16.3% of the patients. RA developed in 8.7% of the patients. By multivariate logistic regression analysis, age ≤40 at disease presentation, non-adherence to therapy and PIP joints involvement were the only factors which independently predicted the risk of treatment failure. Conclusions: Tight control strategy by using DMARDs may control PR and prevent disease progression to RA.(AU)

Objetivos: El reumatismo palindrómico (PR) se caracteriza por episodios repetitivos y afebriles de artritis aguda y periartritis. El objetivo de este estudio fue considerar los resultados a largo plazo de los pacientes con PR que fueron tratados con una estrategia de control estricta utilizando fármacos antirreumáticos modificadores de la enfermedad (DMARD). Métodos: Revisamos los cuadros de 106 pacientes diagnosticados con PR que fueron remitidos al Centro de Investigación de Enfermedades de Tejido Conectivo (CTDRC). Reclutamos a todos los pacientes diagnosticados con PR según los criterios de Hannonen. Visitaron la clínica de CTDRC regularmente y fueron tratados con hidroxicloroquina y prednisolona a dosis bajas debido a episodios activos de PR. En los casos en que los ataques no se controlaron en 3 a 6 meses, se agregó o reemplazó metotrexato y la dosis se aumentó hasta 25mg/semana. En casos resistentes, se añadió sulfasalazina, seguido de la adición de leflunomida y luego azatioprina. El resultado de la enfermedad se evaluó obteniendo la remisión completa o parcial y la prevención de la evolución de la enfermedad a la artritis reumatoide (AR) u otras enfermedades inflamatorias del tejido conectivo. Resultados: Este estudio incluyó 92 pacientes con PR que fueron tratados con DMARD. Los ataques fueron controlados total o parcialmente en 76 (82,6%) pacientes. La remisión libre de medicamentos se obtuvo en el 16,3% de los pacientes. La AR se desarrolló en el 8,7% de los pacientes. Mediante el análisis de regresión logística multivariante, la edad ≤40 en la presentación de la enfermedad, la no adhesión al tratamiento y la afectación de las articulaciones PIP fueron los únicos factores que predijeron de forma independiente el riesgo de fracaso del tratamiento. Conclusiones: Una estrategia de control estricta mediante el uso de DMARD puede controlar la RP y prevenir la progresión de la enfermedad a AR.(AU)

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OBJECTIVES: Palindromic rheumatism (PR) is characterized by repetitive, afebrile episodes of acute arthritis and peri-arthritis. The aim of this study was considering the long-term outcomes of patients with PR who were treated with tight control strategy using Disease-modifying anti-rheumatic drugs (DMARDs). METHODS: We reviewed the charts of 106 patients diagnosed with PR who were referred to the Connective Tissue Diseases Research Center (CTDRC). We recruited all the patients diagnosed with PR according to the criteria of Hannonen. They visited the CTDRC clinic regularly and were treated with hydroxychloroquine and low dose prednisolone because of active episodes of PR. In cases that the attacks did not come under control in 3-6 months, methotrexate was added or replaced and the dose was increased up to 25mg/week. In resistant cases, sulfasalazine was added, followed by the addition of leflunomide and then azathioprine. Disease outcome was evaluated by getting complete or partial remission and prevention of disease evolution to rheumatoid arthritis (RA) or other inflammatory connective tissue diseases. RESULTS: This study included 92 patients with PR who were treated with DMARDs. Attacks were controlled completely or partially in 76 (82.6%) patients. Medications free remission was obtained in 16.3% of the patients. RA developed in 8.7% of the patients. By multivariate logistic regression analysis, age ≤40 at disease presentation, non-adherence to therapy and PIP joints involvement were the only factors which independently predicted the risk of treatment failure. CONCLUSIONS: Tight control strategy by using DMARDs may control PR and prevent disease progression to RA.

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We report the case of a 60-years-old man who complained fever and left knee pain. Fever and arthralgia appear once a month, and these symptoms disappear spontaneously in 3 days. The patient came to our hospital to receive Kampo treatment, because the patient experienced improving these symptoms by Kampo medicine about 20 years ago. I judged the arthralgia as kanshippi, because the arthralgia improved when the patient warmed the joint. We prescribed keishikajutsubuto 7.5 g/day. Arthralgia improved after administration of keishikajutsubuto, but the attack appeared every month. We prescribed maobushisaishinto 5 g/day in addition to keishikajutsubuto 5 g/day for further improvement of arthralgia. The frequency of fever and arthralgia attack was dramatically decreased after we prescribed keishikajutsubuto 5 g/day and maobushisaishinto 5 g/day. We considered the possibility of palindromic rheumatism based on clinical symptoms, X-ray in other clinic and blood test results in our hospital. We report an effective case of keishikajutsubuto and maobushisaishinto on arthritis attacks with fever that resolved spontaneously in a short time.

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OBJECTIVE: This study evaluated anti-modified citrullinated vimentin (anti-MCV) performance in determining the clinical picture and outcomes of palindromic rheumatism (PR). METHODS: In a retrospective study, patients with PR with at least 1 year of follow-up diagnosed according to clinical criteria were enrolled. Anti-MCV antibodies were measured, and levels >20 IU/mL were considered positive. Disease prognosis was assessed according to patients acquiring remission and preventing PR from developing into rheumatoid arthritis (RA) or other diseases. RESULTS: Seventy-six patients with PR with a mean follow-up of 30.57 months (median = 21 months; minimum = 12 months; maximum = 48 months) were included in the study. Anti-MCV antibodies were positive in 69.7% of patients. Metacarpophalangeal (MCP) joint involvement and positive anti-cyclic citrullinated peptides were significantly higher in patients who were anti-MCV-positive, whereas ankle joint involvement was significantly lower. No significant correlation was observed between the anti-MCV titer and the severity of attacks. Remission in patients who were anti-MCV-positive and negative was 75.5% and 78.3%, respectively, with no significant difference. Evolution to RA was observed in only 3.8% of patients who were anti-MCV-positive. No patients who were anti-MCV-negative developed RA. CONCLUSION: Except for MCP and ankle joint involvement, anti-MCV was not helpful in determining the clinical picture and outcome of PR.

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OBJECTIVES: The spectrum of progression of palindromic rheumatism (PR) to chronic diseases is quite variable. Hence, this study aimed to investigate the incidence and risk of developing rheumatic diseases in PR using nationwide, population-based medical claims data from Korea. METHODS: We assessed the incidence rate (IR) of PR in the population in the given year. After matching individuals with PR with those without PR (1:10) for age, gender, and the index year, we calculated the hazard ratios (HRs) with 95% confidence intervals (CIs) using the Cox proportional hazard model. RESULTS: A total of 19,724 newly diagnosed incident PR cases were identified from 2010 to 2016. The mean age was 50.2±14.9 years. The incidence of PR was 7.02 (95% CI 6.92-7.12) per 100,000 person-years (6.22 and 7.80 in men and women, respectively). During observation, 8.79% patients with PR and 0.30% individuals without PR developed various outcome diseases. Patients with PR had an increased risk of seropositive rheumatoid arthritis (HR 46.51, 95% CI [41.05-52.69]), psoriatic arthritis (44.79 [15.16-132.35]), systemic lupus erythematosus (24.53 [16.15-37.24]), mixed connective tissue disease (22.01 [7.65-63.34]), Behçet's disease (21.04 [13.81-32.06]), Sjögren's syndrome (12.36 [8.54-17.88]), ankylosing spondylitis (9.00 [6.67-12.15]), dermatomyositis/polymyositis (6.14 [2.55-14.82]), and systemic sclerosis (3.75 [1.47-9.58]) compared with individuals without PR. CONCLUSIONS: This nationwide, population-based cohort study demonstrated that about one-eleventh of patients with PR eventually develop systemic rheumatic diseases and that patients with PR have an increased risk of developing various rheumatic diseases including seropositive rheumatoid arthritis.

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BACKGROUND: A restricted response against citrullinated peptides/proteins, with less isotype usage, has been found in palindromic rheumatism (PR) in comparison with rheumatoid arthritis (RA). We hypothesized that this different antibody response may be observed for other post-translational modified proteins. We compared the prevalence and isotype usage of two specificities of anti-carbamylated peptide/protein antibodies (Anti-CarP) in patients with PR and RA. METHODS: Cross-sectional study including 54 patients with pure PR and 53 patients with RA, matched by sex, age, disease duration and ACPA. Anti-CarP specificities were determined by home-made enzyme-linked immunosorbent assay tests using a synthetic chimeric fibrin/filaggrin homocitrullinated peptide (CFFHP) and fetal calf serum (FCS) homocitrullinated protein as antigens. IgG, IgA and IgM isotypes were measured. RESULTS: Anti-CarP were positive (CFFHP or FCS) in 24% and 64% of patients with PR and RA, respectively (p < 0.005). All Anti-CarP isotype proportions were significantly lower in PR than in RA: Anti-CarP-IgG (24% versus 51%), Anti-CarP-IgA (7% versus 34%) and Anti-CarP-IgM (7% versus 36%). Mean titers of Anti-CarP isotypes were also lower in PR. In Anti-CarP positive patients, the isotype distribution differed between PR and RA: IgG Anti-CarP was used in all PR patients and in 79% of RA patients. By contrast, a significantly lower isotype usage of both IgA (31% versus 53%) and IgM (31% versus 56%) was observed in PR patients. No significant differences in clinical or demographic characteristics were observed according to Anti-CarP status in PR patients, except for a higher prevalence of ACPA and higher mean titers of ACPA and rheumatoid factor in Anti-CarP positive patients. CONCLUSION: Anti-CarP are found in patients with PR but in a lower proportion and with a different isotype usage from in RA, suggesting a distinct B cell response to homocitrullinated antigens in PR.